Russell-Silver chirwere

Russell-Silver syndrome (RSS) chirwere chiripo pakuzvarwa chinosanganisira kukura kwakashata. Rumwe rutivi rwemuviri runogona zvakare kuratidzika kunge rwakakura kupfuura rwumwe.

Mumwe muvana gumi ane chirwere ichi ane dambudziko rinosanganisira chromosome 7. Mune vamwe vanhu vane chirwere ichi, zvinogona kukanganisa chromosome 11.

Kazhinji yenguva, inoitika muvanhu vasina nhoroondo yemhuri yechirwere.

Huwandu hunofungidzirwa hwevanhu vanovandudza mamiriro aya anosiyana zvakanyanya. Vanhurume nevanhukadzi vanobatwa zvakaenzana.

Zviratidzo zvinogona kusanganisira:

• Mabirthmark ayo ari iwo mavara ekofi nemukaka (cafe-au-lait mamaki)
• Musoro muhombe wekukura kwemuviri, pahuma paupamhi nekakona kadiki-kakasungwa kumeso uye diki, yakatetepa chin
  
• Kukombama kwepinki kwakanangana nemunwe wemhete
• Kukundikana kubudirira, kusanganisira kunonoka zera remapfupa
• Kurema kwekuzvarwa uremu
• Kureba kupfupi, maoko mapfupi, zvigunwe zvinokamhina nezvigunwe
• Dumbu uye matambudziko ematumbo senge acid Reflux uye kupatira

Mamiriro acho anowanzo kuongororwa nehucheche hudiki. Anopa hutano anoita bvunzo dzepanyama.

Iko hakuna chaiwo marabhoritari bvunzo yekuongorora RSS. Kuongororwa kunowanzoenderana nekutongwa kwemupi wemwana wako. Nekudaro, bvunzo dzinotevera dzinogona kuitwa:

• Ropa shuga (vamwe vana vanogona kunge vane shuga shoma yeropa)
• Kuongorora zera repfupa (zera remabhonzo rinowanzo kuve diki pane zera chairo remwana)
• Genetic kuyedza (inogona kuona chinetso chromosomal)
• Kukura kwehomoni (vamwe vana vanogona kushomeka)
• Skeletal ongororo (kutonga kunze mamwe mamiriro anogona kutevedzera RSS)

Kukura kwehomoni kutsiva kunogona kubatsira kana iyi hormone isipo. Mimwe mishonga inosanganisira:

• Kuita shuwa kuti munhu anowana macalorie akakwana ekudzivirira yakaderera ropa shuga uye kusimudzira kukura
• Kurapa kwepanyama kuvandudza mhasuru toni
• Rubatsiro pakudzidza kugadzirisa hurema pakudzidza uye matambudziko ekushomeka kwekutarisa anogona kunge aine mwana

Nyanzvi zhinji dzinogona kuve nechekuita pakurapa munhu ane chirwere ichi. Zvinosanganisira:

• Chiremba anoongorora genetics kubatsira kuongorora RSS
• Gastroenterologist kana chiremba wezvekudya kuti abatsire kugadzira kudya kwakakodzera kusimudzira kukura
• An endocrinologist yekuraira kukura mahormone
• A genetic chipangamazano uye wezvepfungwa

Vana vakura nevakuru havaratidze chaiwo maficha zvakajeka sevacheche kana vana vadiki. Kungwara kunogona kuve kwakajairika, kunyangwe munhu angave aine hurema hwekudzidza.Kuremara kwekuzvarwa kweturakiti kunogona kunge kuripo.

Vanhu vane RSS vanogona kunge vaine matambudziko aya:

• Kutsenga kana kutaura kunetsa kana shaya iri diki kwazvo
• Kuremara pakudzidza

Dana mupi wemwana wako kana zviratidzo zveRSS zvichikura. Ita shuwa kuti urefu uye huremu hwemwana wako zvinoyerwa panguva yega yega yekushanya-kwemwana. Iye mupi anogona kukuendesa kune:

• A genetic nyanzvi yeongororo yakazara uye chromosome zvidzidzo
• Yevana endocrinologist yekutarisira matambudziko emwana wako ekukura

Silver-Russell chirwere; Silver chirwere; RSS; Russell-Silver chirwere

Haldeman-Englert CR, Saitta SC, Zackai EH. Chromosome matambudziko. Mune: Gleason CA, Juul SE, eds. Zvirwere zveAvery zveMucheche. 10th ed. Philadelphia, PA: Elsevier; 2018: chitsiko 20.

Wakeling EL, Brioude F, Lokulo-Sodipe O, et al. Kuongororwa uye manejimendi yeSilver-Russell syndrome: yekutanga nyika chibvumirano chirevo. Nat Rev Endocrinol. 2017; 13 (2): 105-124. PMID: 27585961 pubmed.ncbi.nlm.nih.gov/27585961/.